Stock 129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

FESA Number


Strain Name

129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

Mutation Strain Type


Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Prnp<tm1Cwe> MGI:1888773 Prnp MGI:97769 2

Phenotypic Description

The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.

Orphanet Disorders

OrphaNet ID Name Url
356 Gerstmann-Straussler-Scheinker syndrome
466 Fatal familial insomnia
157 941 Huntington disease-like 1
280 397 Familial Alzheimer-like prion disease
282 166 Inherited Creutzfeldt-Jakob disease


Displaying 31 - 40 of 226 Stock Publications
Pubmed ID Authors Title Journal
24329154 Alvarez-Laviada A The inhibition of functional expression of calcium channels by prion protein demonstrates competition with ?2? for GPI-anchoring pathways. Biochem J (2 014) 458:365-74
22428057 Biasini E The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells. PLoS One (2 012) 7:e33472
20472884 Christensen HM A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death. Am J Pathol (2 010) 176:2695-706
16621165 Diez M Changes in neuropeptide expression in mice infected with prions. Neurobiol Aging (2 007) 28:748-65
16738485 Gains MJ Prion protein protects against ethanol-induced Bax-mediated cell death in vivo. Neuroreport (2 006) 17:903-6
17420473 Huang S Inducible overexpression of wild-type prion protein in the muscles leads to a primary myopathy in transgenic mice. Proc Natl Acad Sci U S A (2 007) 104:6800-5
12878182 Kubosaki A Expression of normal cellular prion protein (PrP(c)) on T lymphocytes and the effect of copper ion: Analysis by wild-type and prion protein gene-deficient mice. Biochem Biophys Res Commun (2 003) 307:810-3
15694261 Lobão-Soares B Normal brain mitochondrial respiration in adult mice lacking cellular prion protein. Neurosci Lett (2 005) 375:203-6
18514536 Medrano AZ GFP-tagged mutant prion protein forms intra-axonal aggregates in transgenic mice. Neurobiol Dis (2 008) 31:20-32
22860039 Passet B Prion protein and Shadoo are involved in overlapping embryonic pathways and trophoblastic development. PLoS One (2 012) 7:e41959


Orphanet Categories

Rare neurologic disease



Genetic Status


Background Strain Name


Background Strain MGI ID



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